Animal models of primary biliary cirrhosis: Materials and methods

Patrick S Leung, Guo Xiang Yang, Amy Dhirapong, Koichi Tsuneyama, William M. Ridgway, M. Eric Gershwin

Research output: Chapter in Book/Report/Conference proceedingChapter

25 Scopus citations


Primary biliary cirrhosis (PBC) is a female-predominant autoimmune disease of the liver characterized by immune-mediated destruction of the intrahepatic bile ducts and the presence of antimitochondrial antibodies (AMAs). There have been limited advances in understanding the molecular pathogenesis of the disease because of the difficulty in accessing human tissues and the absence of appropriate animal models. Recently, several unique murine models that manifest the serological, biochemical, and histological features similar to human PBC have been described. In this article, we discuss the current data on three spontaneous and two induced murine models of PBC. The spontaneous models are: (a) NOD.c3c4, (b) dominant negative TGF-β receptor II (dnTGFβRII), and (c) IL-2Rα-/- mouse line models. The two induced models are: (a) xenobiotic and (b) Novosphingobium aromaticivorans immunized mice. These animal models provide various important platforms to further investigate the etiology and mechanisms of pathogenesis in PBC. Laboratory methodologies and the protocols that are used in evaluating these animal models are described. Finally, we stress the importance of realizing the strengths and limitations of the animal models are essential in data analysis and their application in therapeutic studies.

Original languageEnglish (US)
Title of host publicationMethods in Molecular Biology
Number of pages26
StatePublished - 2012

Publication series

NameMethods in Molecular Biology
ISSN (Print)10643745


  • Antimitochondrial antibodies
  • Biliary epithelial cells
  • Cytokine analysis
  • E2 subunit of pyruvate dehydrogenase
  • Flow cytometry
  • Immunohistochemical staining
  • Liver lesions
  • Primary biliary cirrhosis
  • Western Blot

ASJC Scopus subject areas

  • Molecular Biology
  • Genetics


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